Ryszard Żaba

Eosinophilic fascitis: a report of two cases treated with ultraviolet A1 phototherapy.

Eosinophilic fascitis (EF) (synonyms: Shulmans syndrome, diffuse fascitis with eosinophilia) is a disease characterized by a complex set of symptoms with scleroderma‐like skin lesions, the absence of Raynauds phenomenon and other non‐mandatory symptoms including eosinophilia, elevated erythrocyte sedimentation rate, hypergammaglobulinemia and high levels of circulating immune complexes. EF is probably not a separate disease entity, but an acute variant of localized scleroderma. This rare disease of unknown etiology is usually seen in middle‐aged adults. Sclerodermiform indurations without Raynauds symptoms develop rapidly usually on the extremities and more rarely on the trunk or the face. The skin becomes hard, tightly bound to the underlying structures, so that contractures can develop in as little as a few weeks. The course of the disease is usually chronic but spontaneous remission is possible. Standard therapy includes high doses of corticosteroids, immunosuppressive drugs such as methotrexate, cyclosporin A, cyclophosphamide or azathioprine and others such as psoralen and ultraviolet A radiation.

Current views on the etiopathogenesis, clinical manifestation, diagnostics, treatment and correlation with other nosological entities of SIBO

Small intestinal bacterial overgrowth (SIBO) is a disease of great clinical and socioeconomic importance caused by an excessive amount of bacteria in the upper alimentary tract. Physiological microbiota are replaced by pathogenic bacteria mainly from large intestine, which is called dysbacteriosis. SIBO disturbs digestion and absorption in the alimentary tract, which seems to cause inflammation. SIBO affects the morphology and function of the digestive system and causes systemic complications (e.g. osteoporosis, macrocytic anemia). Inflammation interferes with gene expression responsible for producing and secreting mucus, therefore, a correlation between SIBO and cystic fibrosis, irritable bowel syndrome and chronic abdominal pain are postulated. All conditions leading to bacterial growth such as congenital and anatomical abnormalities in the digestive tract, motility disorder or immunological deficits are risk factors of SIBO. A typical clinical manifestation of SIBO comprises meteorism, enterectasia, abdominal discomfort and diarrhea. Diagnostic procedures such as glucose, lactulose, methane, 13C mixed triglyceride breath tests are being used in diagnosing SIBO.

Seemingly healthy skin in atopic dermatitis: observations with the use of high-frequency ultrasonography, preliminary study.

Background: Atopic dermatitis (AD) is a chronic, relapsing skin disorder which is strictly determined by the epidermal barrier function. In previous studies, there is conclusive evidence that normal‐looking, nonlesional skin presents meaningful barrier function defect and a sub‐clinical eczematous skin reaction.

Expression of selected human endogenous retroviral sequences in skin and peripheral blood mononuclear cells in morphea.

Introduction Morphea or localized scleroderma is a relatively rare disease whose main symptom is excessive skin fibrosis. Here we focus on the involvement of human endogenous retroviruses (HERVs) in morphea. The HERVs are a vast and intensely growing field in genomics. HERVs are of special interest as far as autoimmune disorders are concerned, yet little effort has been made until now to assess the possible changes of their expression in morphea. Material and methods Six sequences of particular interest were chosen for this study. Real-time polymerase chain reaction was performed on samples derived from peripheral blood mononuclear cells (PBMCs) and skin biopsies. The results were normalized to the level of glyceraldehyde 3-phosphate dehydrogenase (GAPDH) transcription. Results In PBMCs we found a statistically significant decrease of transcription of HERV-E pol, while HERV-K env, HERV-R pol-env, and HERV-W env were found to be up-regulated. In skin biopsies HERV-K env was strongly up-regulated. On the other hand, we noted a decrease of transcription of HERV-H env 62, HERV-K10 gag, HERV-R pol-env, and HERV-W env. In PBMCs we found a statistically significant decrease of transcription of HERV-E pol (–81.8%, p < 0.001), while HERV-K env (+94.1%, p = 0.010), HERV-R pol-env (+140.0%, p < 0.001), and HERV-W env (+97.7%, p < 0.001) were found to be up-regulated. In skin biopsies HERV-K env was strongly up-regulated (+713.0%, p = 0.003). On the other hand, we noted a decrease of transcription of HERV-H env 62 (–83.5%, p < 0.001, HERV-K10 gag (-33.7%, p = 0.044), HERV-R pol-env (–71.3%, p < 0.001), and HERV-W env (–59.3%, p = 0.029). Conclusions The studied HERV sequences generally show an increase of transcription in PBMCs of morphea patients, while being down-regulated in their skin, with some exceptions for both types of tissue.

Inhibitors of phosphodiesterase 4 (PDE 4): A new therapeutic option in the treatment of psoriasis vulgaris and psoriatic arthritis

Abstract Psoriasis vulgaris and psoriatic arthritis are inflammatory diseases in which inflammation and sustained inducing lesions result from immune disorders associated with overactivity of T cells that produce multiple proinflammatory cytokines, including tumor necrosis factor alpha (TNF-α) and interleukin (IL): IL-2, IL-12, IL-17, IL-22 or IL-23. Modern treatment of these diseases is focused on reducing the inflammatory process responsible for the development of the disease. In recent years, the treatment of psoriasis is developing at a dynamic rate. Such therapeutic advances are contributed to the possibility of patient therapy through the use of some registered biologic agents, such as TNF-α inhibitors (infliximab, etanercept and adalimumab), and an inhibitor of the p40 subunit common to IL-12 and IL-23 (ustekinumab). In addition to the already registered medications for the indications mentioned above, there is a large group of preparations that are currently undergoing clinical trials in Europe, Canada and the United States, which provides hopes of therapy efficacy and safety.

“Assessment of chronic sclerodermoid Graft-versus-Host Disease patients, using 20MHz high-frequency ultrasonography and cutometer methods”

The development of an adverse graft‐versus‐host disease (GvHD) is a major complication of stem cell transplantations, which are widely used to cure increasing number of hematologic malignancies. Patients with chronic GvHD are at risk of joint contractures secondary to sclerodermatous skin changes. Several clinical scores or serologic markers have been used to assess skin sclerosis in scleroderma patients. Evaluation of sclerotic skin changes using biometric tools remains to be challenging. The purpose of this study was to illustrate and exemplify ultrasound measurement and measurement of skin elasticity of five chronic sclerodermoid GvHD patients. There is still a substantial lack of studies using objective and non‐invasive methods helpful in assessment of patients with skin involvement of GvHD. Although ultrasound is not the ideal method, it is worth emphasizing that it is still useful, non‐invasive, and repeatable device in monitoring patients suffering from GvHD. It should also be added, that it seems to be advisable to repeat USG examination at an interval of 3 months after the treatment. In addition, skin echogenicity may be a more sensitive parameter than skin thickness in assessment of cGvHD patients.

Gardner-Diamond syndrome.

The Gardner–Diamond syndrome (GDS), also known as autoerythrocyte sensitization syndrome, painful bruising syndrome, and psychogenic purpura, is a rare autoimmune vasculopathy. People with it react against a component of their own erythrocytes, most likely phosphatidylserine, a phosphoglyceride of the cell membrane. In most cases, it is diagnosed among women younger than 30 years old, and is characterized by painful ecchymoses. It tends to develop after severe stress, emotional trauma, or mental disease. The diagnosis is based upon painful erythematous patches progressing to ecchymoses within 24 h and a positive diagnostic test with intracutaneous injections of 80% solution of washed autologous erythrocytes. Hematologic studies, including coagulation parameters, are usually normal with a skin biopsy specimen showing only nonspecific changes. The autoerythrocyte sensitization syndrome has been rarely described as the primary manifestation of systemic lupus erythematosus. We present the case of a 23-year-old female student with GDS who had an ANA titer of 1/320 with anti-double stranded DNA and review the literature.

The role of altered cutaneous immune responses in the induction and persistence of rosacea.

Rosacea is a chronic inflammatory skin condition that predominantly affects the skin of the face and the eyes. Several factors are associated with the onset and persistence of the condition, including an altered immune response in the skin and elevated levels of Demodex mites. Alterations in the immune response include elevated levels of LL-37 in rosacea skin, increased expression of TLR-2 and increased amounts of vitamin D3 in epidermal tissue. The combined effect of these changes may make the skin more sensitive to external and internal stimuli. External stimuli that may trigger or sustain rosacea inflammation include exposure to ultraviolet light, while internal factors may include the presence of elevated numbers of Demodex mites. These mites may directly stimulate an immune response or release bacteria within the pilosebaceous unit that act as a trigger for inflammation. This review will highlight the changes that occur in the immune response of the skin and describe how Demodex mites and associated bacteria may activate this response and lead to the characteristics of rosacea.

Health-related quality of life, optimism, and coping strategies in persons suffering from localized scleroderma

The clinical course of localized scleroderma may consist of bodily deformations, and bodily functions may also be affected. Additionally, the secondary lesions, such as discoloration, contractures, and atrophy, are unlikely to regress. The aforementioned symptoms and functional disturbances may decrease one’s quality of life (QoL). Although much has been mentioned in the medical literature regarding QoL in persons suffering from dermatologic diseases, no data specifically describing patients with localized scleroderma exist. The aim of the study was to explore QoL in localized scleroderma patients and to examine their coping strategies in regard to optimism and QoL. The study included 41 patients with localized scleroderma. QoL was evaluated using the SKINDEX questionnaire, and levels of dispositional optimism were assessed using the Life Orientation Test-Revised. In addition, individual coping strategy was determined using the Mini-MAC scale and physical condition was assessed using the Localized Scleroderma Severity Index. The mean QoL score amounted to 51.10 points, with mean scores for individual components as follows: symptoms = 13.49 points, emotions = 21.29 points, and functioning = 16.32 points. A relationship was detected between QoL and the level of dispositional optimism as well as with coping strategies known as anxious preoccupation and helplessness–hopelessness. Higher levels of optimism predicted a higher general QoL. In turn, greater intensity of anxious preoccupied and helpless–hopeless behaviors predicted a lower QoL. Based on these results, it may be stated that localized scleroderma patients have a relatively high QoL, which is accompanied by optimism as well as a lower frequency of behaviors typical of emotion-focused coping strategies.

Quality of Life and Optimism in Patients with Morphea

Despite extensive knowledge about quality of life of people suffering from dermatological diseases, data on patients with morphea are scarce. The aim of our study was to compare the quality of life of healthy controls and morphea patients, as well as to determine the correlation of this variable with the level of dispositional optimism. The study included 47 patients with morphea and 47 healthy controls, matched for gender and age. Cantril’s Ladder and Life Orientation Test-Revised were used to assess the levels of life satisfaction and dispositional optimism, respectively. LoSSI was used for the objective assessment. The anticipated level of life quality and the level of dispositional optimism were statistically significantly lower in morphea patients (p = 0.032 and p = 0.014, respectively) when compared to controls. There were no differences in the assessment of current (p = 0.168) and past (p = 0.318) levels of life quality. Also, we proved that type of morphea did not differentiate the current (p = 0.175), past (p = 0.620) and future (p = 0.356) assessment of the quality of life. In the group of morphea patients there was a statistically significant correlation between the level of dispositional optimism and current (p = 0.002, r = 0.43), as well as anticipated (p < 0.001, r = 0.57) levels of life quality. Current level of life quality of healthy controls and morphea patients is comparable, whereas the latter anticipate their future life situation to be significantly worse than the former. Higher level of life satisfaction correlates with higher level of optimism.